review article | Q7318358 |
scholarly article | Q13442814 |
P356 | DOI | 10.1007/S10863-010-9288-5 |
P698 | PubMed publication ID | 20464463 |
P2093 | author name string | Kochupurackal P Mohanakumar | |
Mritunjay Pandey | |||
Rajamma Usha | |||
P2860 | cites work | Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator | Q24294798 |
Huntingtin promotes cell survival by preventing Pak2 cleavage | Q24314724 | ||
Towards a transgenic model of Huntington's disease in a non-human primate | Q24644013 | ||
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. | Q27860836 | ||
Inhibition of calpain cleavage of huntingtin reduces toxicity: accumulation of calpain/caspase fragments in the nucleus | Q28505053 | ||
Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survival | Q28580488 | ||
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro | Q28584697 | ||
Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease | Q28589793 | ||
A Huntington's disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice | Q28594336 | ||
Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice | Q29615357 | ||
Early degenerative changes in transgenic mice expressing mutant huntingtin involve dendritic abnormalities but no impairment of mitochondrial energy production | Q45302993 | ||
Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice | Q45304684 | ||
Clinical correlates of mitochondrial function in Huntington's disease muscle | Q45305337 | ||
Cytochrome C and caspase-9 expression in Huntington's disease | Q45306362 | ||
Mitochondrial NAD+-linked State 3 respiration and complex-I activity are compromised in the cerebral cortex of 3-nitropropionic acid-induced rat model of Huntington's disease. | Q45306770 | ||
Oxidative stress parameters in plasma of Huntington's disease patients, asymptomatic Huntington's disease gene carriers and healthy subjects : a cross-sectional study | Q45306906 | ||
Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington's disease. | Q45307199 | ||
DNA End Labeling (TUNEL) in Huntington's Disease and Other Neuropathological Conditions | Q45307545 | ||
Caspase-dependent and -independent cell death induced by 3-nitropropionic acid in rat cortical neurons. | Q46859922 | ||
Expression of the Huntington's disease gene is regulated in astrocytes in the arcuate nucleus of the hypothalamus of postpartum rats | Q48204539 | ||
Functional anatomy of the basal ganglia. I. The cortico-basal ganglia-thalamo-cortical loop | Q56093340 | ||
Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid | Q72098398 | ||
Mitochondrial aconitase is a source of hydroxyl radical. An electron spin resonance investigation | Q73754845 | ||
Mitochondrial swelling impairs the transport of organelles in cerebellar granule neurons | Q81038733 | ||
Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization | Q95810356 | ||
Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction. | Q30164317 | ||
Characterization of cybrid cell lines containing mtDNA from Huntington's disease patients. | Q30304195 | ||
Relationships among molecular genetic and respiratory properties of Parkinson's disease cybrid cells show similarities to Parkinson's brain tissues | Q30437451 | ||
Reduced axonal transport in Parkinson's disease cybrid neurites is restored by light therapy | Q30439165 | ||
Chronic, low-dose rotenone reproduces Lewy neurites found in early stages of Parkinson's disease, reduces mitochondrial movement and slowly kills differentiated SH-SY5Y neural cells | Q30440337 | ||
N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking | Q30486372 | ||
Effects of overexpression of huntingtin proteins on mitochondrial integrity | Q30489322 | ||
Inhibitors of cytochrome c release with therapeutic potential for Huntington's disease | Q30847955 | ||
Apoptotic mode of cell death in substantia nigra following intranigral infusion of the parkinsonian neurotoxin, MPP+ in Sprague-Dawley rats: cellular, molecular and ultrastructural evidences | Q33280786 | ||
Unraveling a role for dopamine in Huntington's disease: the dual role of reactive oxygen species and D2 receptor stimulation | Q33922831 | ||
Loss of mitochondrial membrane potential is associated with increase in mitochondrial volume: physiological role in neurones. | Q33989454 | ||
Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates | Q34103541 | ||
Glutamate transporters in neurologic disease | Q34187611 | ||
Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid | Q34384772 | ||
Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin | Q34483577 | ||
Ca(2+) signalling in mitochondria: mechanism and role in physiology and pathology. | Q35196252 | ||
Reactive oxygen species and the central nervous system | Q35389986 | ||
Selective defect of in vivo glycolysis in early Huntington's disease striatum | Q35634060 | ||
Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease | Q35706627 | ||
Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats | Q35882893 | ||
Sustained elevation of extracellular dopamine causes motor dysfunction and selective degeneration of striatal GABAergic neurons. | Q35978877 | ||
Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity | Q36320596 | ||
Regulation of mitochondrial matrix volume | Q36548160 | ||
Aggregation of huntingtin in yeast varies with the length of the polyglutamine expansion and the expression of chaperone proteins | Q37109961 | ||
Impaired PGC-1alpha function in muscle in Huntington's disease. | Q37323316 | ||
Mitochondrial structural and functional dynamics in Huntington's disease | Q37356257 | ||
Impaired mitochondrial trafficking in Huntington's disease | Q37466100 | ||
Duplication of biochemical changes of Huntington's chorea by intrastriatal injections of glutamic and kainic acids | Q39114293 | ||
Mitochondrial-dependent apoptosis in Huntington's disease human cybrids. | Q39751441 | ||
Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease | Q40031554 | ||
Ubiquitin-proteasome system alterations in a striatal cell model of Huntington's disease | Q40141779 | ||
Extended polyglutamine repeats trigger a feedback loop involving the mitochondrial complex III, the proteasome and huntingtin aggregates | Q40159836 | ||
Increased oxidative damage and mitochondrial abnormalities in the peripheral blood of Huntington's disease patients | Q40207281 | ||
Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences | Q40231756 | ||
Oxidative stress promotes mutant huntingtin aggregation and mutant huntingtin-dependent cell death by mimicking proteasomal malfunction. | Q40317881 | ||
Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation | Q40351019 | ||
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release | Q40551652 | ||
Huntington's disease, energy, and excitotoxicity | Q40580788 | ||
Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. | Q40721588 | ||
Calpain activation in Huntington's disease. | Q40723358 | ||
Altered proteasomal function due to the expression of polyglutamine-expanded truncated N-terminal huntingtin induces apoptosis by caspase activation through mitochondrial cytochrome c release | Q40808988 | ||
Huntingtin's neuroprotective activity occurs via inhibition of procaspase-9 processing | Q40817529 | ||
A cellular model that recapitulates major pathogenic steps of Huntington's disease | Q41017315 | ||
Regional mitochondrial respiratory activity in Huntington's disease brain | Q41360902 | ||
Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease | Q41698021 | ||
Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway. | Q41969689 | ||
Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia. | Q42438521 | ||
Widespread expression of the human and rat Huntington's disease gene in brain and nonneural tissues | Q42503942 | ||
Metabolic changes in the basal ganglia of patients with Huntington's disease: an in situ hybridization study of cytochrome oxidase subunit I mRNA. | Q42518943 | ||
Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II. | Q43206399 | ||
L-DOPA-induced 6-hydroxydopamine production in the striata of rodents is sensitive to the degree of denervation | Q43237341 | ||
Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease | Q43830045 | ||
Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice | Q44316963 | ||
The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis. | Q44376761 | ||
Calpain is a major cell death effector in selective striatal degeneration induced in vivo by 3-nitropropionate: implications for Huntington's disease. | Q44495460 | ||
In vivo calpain/caspase cross-talk during 3-nitropropionic acid-induced striatal degeneration: implication of a calpain-mediated cleavage of active caspase-3. | Q44548881 | ||
Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila | Q44607268 | ||
Proteomic and oxidative stress analysis in human brain samples of Huntington disease. | Q44737699 | ||
Mitochondrial calcium, oxidative stress and apoptosis in a neurodegenerative disease model induced by 3-nitropropionic acid | Q44792354 | ||
Mitochondrial defect in Huntington's disease caudate nucleus | Q45291734 | ||
In situ mitochondrial Ca2+ buffering differences of intact neurons and astrocytes from cortex and striatum. | Q45292096 | ||
Cytochrome c oxidase isoform IV-2 is involved in 3-nitropropionic acid-induced toxicity in striatal astrocytes | Q45293168 | ||
Striatal dopamine level contributes to hydroxyl radical generation and subsequent neurodegeneration in the striatum in 3-nitropropionic acid-induced Huntington's disease in rats | Q45293625 | ||
Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. | Q45293805 | ||
Evidence for a defect in NADH: ubiquinone oxidoreductase (complex I) in Huntington's disease | Q45294442 | ||
Coenzyme Q10 serum levels in Huntington's disease | Q45294694 | ||
Complex I defect in muscle from patients with Huntington's disease. | Q45295738 | ||
Huntingtin protein colocalizes with lesions of neurodegenerative diseases: An investigation in Huntington's, Alzheimer's, and Pick's diseases | Q45295797 | ||
Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. | Q45296558 | ||
Dopamine modulates the susceptibility of striatal neurons to 3-nitropropionic acid in the rat model of Huntington's disease. | Q45296860 | ||
Biochemical abnormalities and excitotoxicity in Huntington's disease brain. | Q45297145 | ||
Neuropathological classification of Huntington's disease | Q45297167 | ||
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin | Q45297223 | ||
Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin | Q45297269 | ||
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. | Q45298582 | ||
Effect of coenzyme Q10 and vitamin E on brain energy metabolism in the animal model of Huntington's disease | Q45299091 | ||
Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortex | Q45299186 | ||
Striatal modulation of cAMP-response-element-binding protein (CREB) after excitotoxic lesions: implications with neuronal vulnerability in Huntington's disease. | Q45299845 | ||
Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG. | Q45299923 | ||
Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons | Q45300079 | ||
Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity. | Q45300254 | ||
Oxidative stress in skin fibroblasts cultures of patients with Huntington's disease. | Q45302342 | ||
Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. | Q45302702 | ||
Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration | Q45302924 | ||
Mitochondrial-dependent Ca2+ handling in Huntington's disease striatal cells: effect of histone deacetylase inhibitors. | Q45302980 | ||
P433 | issue | 3 | |
P921 | main subject | mitochondrion | Q39572 |
Huntington's disease | Q190564 | ||
pathophysiology | Q1135939 | ||
P304 | page(s) | 217-226 | |
P577 | publication date | 2010-06-01 | |
P1433 | published in | Journal of Bioenergetics and Biomembranes | Q5044470 |
P1476 | title | Mitochondrial functional alterations in relation to pathophysiology of Huntington's disease | |
P478 | volume | 42 |
Q26853356 | Cell signaling and mitochondrial dynamics: Implications for neuronal function and neurodegenerative disease |
Q45289712 | Degeneration of the cerebellum in Huntington's disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process. |
Q34137813 | Distribution of GABAergic interneurons and dopaminergic cells in the functional territories of the human striatum |
Q57178431 | Huntington's disease: the coming of age |
Q51055442 | Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy. |
Q36124905 | Mitochondria: the next (neurode)generation |
Q37749622 | Mitochondrial matters in Huntington disease |
Q27002338 | Physiological consequences of complex II inhibition for aging, disease, and the mKATP channel |
Q38855572 | Quercetin improves the activity of the ubiquitin-proteasomal system in 150Q mutated huntingtin-expressing cells but exerts detrimental effects on neuronal survivability |
Q37408287 | The Association of VDAC with Cell Viability of PC12 Model of Huntington's Disease. |
Q22306292 | The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease |
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